Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Peutz-Jeghers Syndrome

  • Armagan Gunal
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1667

Synonyms

Periorificial lentiginosis; Polyps and spots syndrome

Definition

Peutz-Jeghers polyposis is a hereditary cancer syndrome which belongs to hamartomatous polyposis syndromes. It is characterized by mucocutaneous melanotic hyperpigmentation and hamartomatous polyps in gastrointestinal tract with an increased risk of intestinal and also extraintestinal malignancies.

Hamartoma defines an overgrowth of normal tissues in their own native localisations. Hamartomatous polyps in gastrointestinal tract can be seen sporadically, but multiple polyps can occur as a component of genetically inherited or acquired hamartomatous polyposis syndromes. Hamartomatous polyposis syndromes include: (1) Juvenile polyposis, (2) Peutz-Jeghers polyposis (PJP), (3) Cowden syndrome and Bannayan-Ruvalcaba-Riley syndrome (these two are also called as “PTEN hamartoma tumor syndrome” because of the same affected gene locus), (4) Cronkhite-Canada syndrome (the latter is different from the former three because of...

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References and Further Reading

  1. Cauchin, E., Touchefeu, Y., & Matysiak-Budnik, T. (2015). Hamartomatous tumors in gastrointestinal tract. Gastrointestinal Tumors, 2, 65–74.CrossRefGoogle Scholar
  2. Hornick, J. L., & Odze, R. D. (2015). Polyps of the large intestine. In R. D. Odze & J. R. Goldblum (Eds.), Surgical pathology of the GI tract, liver, biliary tract and pancreas (pp. 607–655). Philadelphia: Elsevier.Google Scholar
  3. Jung, I., Gurzu, S., & Turdean, G. S. (2015). Current status of familial gastrointestinal polyposis syndromes. World Journal of Gastrointestinal Oncology, 7, 347–355.CrossRefGoogle Scholar
  4. Offerhaus, G. J. A., Billaud, M., & Gruber, S. B. (2010). Peutz-Jeghers syndrome. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (pp. 168–170). Lyon: IARC.Google Scholar
  5. Shaco-Levy, R., Jasperson, K. W., Martin, K., Samadder, N. J., Burt, R. W., Ying, J., & Bronner, M. P. (2016). Morphologic characterization of hamartomatous gastrointestinal polyps in Cowden syndrome, Peutz-Jeghers syndrome and juvenile polyposis syndrome. Human Pathology, 49, 39–48.CrossRefGoogle Scholar
  6. Turner, J. R. (2015). The gastrointestinal tract. In V. Kumar, A. K. Abbas, & J. C. Aster (Eds.), Pathologic basis of disease (pp. 750–817). Philadelphia: Elsevier.Google Scholar

Copyright information

© Springer-Verlag 2017

Authors and Affiliations

  1. 1.Department of PathologyGulhane Military Medical AcademyEtlikTurkey